Hmdb loader

Showing Protein Cartilage oligomeric matrix protein (HMDBP02410)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 1 metabolite
Identification
HMDB Protein ID HMDBP02410
Secondary Accession Numbers
  • 7903
Name Cartilage oligomeric matrix protein
Synonyms
  1. COMP
  2. TSP5
  3. Thrombospondin-5
Gene Name COMP
Protein Type Enzyme
Biological Properties
General Function Involved in calcium ion binding
Specific Function May play a role in the structural integrity of cartilage via its interaction with other extracellular matrix proteins such as the collagens and fibronectin. Can mediate the interaction of chondrocytes with the cartilage extracellular matrix through interaction with cell surface integrin receptors. Could play a role in the pathogenesis of osteoarthritis. Potent suppressor of apoptosis in both primary chondrocytes and transformed cells. Suppresses apoptosis by blocking the activation of caspase-3 and by inducing the IAP family of survival proteins (BIRC3, BIRC2, BIRC5 and XIAP). Essential for maintaining a vascular smooth muscle cells (VSMCs) contractile/differentiated phenotype under physiological and pathological stimuli. Maintains this phenotype of VSMCs by interacting with ITGA7
Pathways Not Available
Reactions Not Available
GO Classification
Component
extracellular region
Function
ion binding
cation binding
metal ion binding
binding
calcium ion binding
Process
cell adhesion
cellular process
Cellular Location
  1. Secreted
  2. extracellular space
  3. extracellular matrix
Gene Properties
Chromosome Location Chromosome:1
Locus 19p13.1
SNPs COMP
Gene Sequence 
>2274 bp
ATGGTCCCCGACACCGCCTGCGTTCTTCTGCTCACCCTGGCTGCCCTCGGCGCGTCCGGA
CAGGGCCAGAGCCCGTTGGGCTCAGACCTGGGCCCGCAGATGCTTCGGGAACTGCAGGAA
ACCAACGCGGCGCTGCAGGACGTGCGGGACTGGCTGCGGCAGCAGGTCAGGGAGATCACG
TTCCTGAAAAACACGGTGATGGAGTGTGACGCGTGCGGGATGCAGCAGTCAGTACGCACC
GGCCTACCCAGCGTGCGGCCCCTGCTCCACTGCGCGCCCGGCTTCTGCTTCCCCGGCGTG
GCCTGCATCCAGACGGAGAGCGGCGGCCGCTGCGGCCCCTGCCCCGCGGGCTTCACGGGC
AACGGCTCGCACTGCACCGACGTCAACGAGTGCAACGCCCACCCCTGCTTCCCCCGAGTC
CGCTGTATCAACACCAGCCCGGGGTTCCGCTGCGAGGCTTGCCCGCCGGGGTACAGCGGC
CCCACCCACCAGGGCGTGGGGCTGGCTTTCGCCAAGGCCAACAAGCAGGTTTGCACGGAC
ATCAACGAGTGTGAGACCGGGCAACATAACTGCGTCCCCAACTCCGTGTGCATCAACACC
CGGGGCTCCTTCCAGTGCGGCCCGTGCCAGCCCGGCTTCGTGGGCGACCAGGCGTCCGGC
TGCCAGCGCGGCGCACAGCGCTTCTGCCCCGACGGCTCGCCCAGCGAGTGCCACGAGCAT
GCAGACTGCGTCCTAGAGCGCGATGGCTCGCGGTCGTGCGTGTGTCGCGTTGGCTGGGCC
GGCAACGGGATCCTCTGTGGTCGCGACACTGACCTAGACGGCTTCCCGGACGAGAAGCTG
CGCTGCCCGGAGCCGCAGTGCCGTAAGGACAACTGCGTGACTGTGCCCAACTCAGGGCAG
GAGGATGTGGACCGCGATGGCATCGGAGACGCCTGCGATCCGGATGCCGACGGGGACGGG
GTCCCCAATGAAAAGGACAACTGCCCGCTGGTGCGGAACCCAGACCAGCGCAACACGGAC
GAGGACAAGTGGGGCGATGCGTGCGACAACTGCCGGTCCCAGAAGAACGACGACCAAAAG
GACACAGACCAGGACGGCCGGGGCGATGCGTGCGACGACGACATCGACGGCGACCGGATC
CGCAACCAGGCCGACAACTGCCCTAGGGTACCCAACTCAGACCAGAAGGACAGTGATGGC
GATGGTATAGGGGATGCCTGTGACAACTGTCCCCAGAAGAGCAACCCGGATCAGGCGGAT
GTGGACCACGACTTTGTGGGAGATGCTTGTGACAGCGATCAAGACCAGGATGGAGACGGA
CATCAGGACTCTCGGGACAACTGTCCCACGGTGCCTAACAGTGCCCAGGAGGACTCAGAC
CACGATGGCCAGGGTGATGCCTGCGACGACGACGACGACAATGACGGAGTCCCTGACAGT
CGGGACAACTGCCGCCTGGTGCCTAACCCCGGCCAGGAGGACGCGGACAGGGACGGCGTG
GGCGACGTGTGCCAGGACGACTTTGATGCAGACAAGGTGGTAGACAAGATCGACGTGTGT
CCGGAGAACGCTGAAGTCACGCTCACCGACTTCAGGGCCTTCCAGACAGTCGTGCTGGAC
CCGGAGGGTGACGCGCAGATTGACCCCAACTGGGTGGTGCTCAACCAGGGAAGGGAGATC
GTGCAGACAATGAACAGCGACCCAGGCCTGGCTGTGGGTTACACTGCCTTCAATGGCGTG
GACTTCGAGGGCACGTTCCATGTGAACACGGTCACGGATGACGACTATGCGGGCTTCATC
TTTGGCTACCAGGACAGCTCCAGCTTCTACGTGGTCATGTGGAAGCAGATGGAGCAAACG
TATTGGCAGGCGAACCCCTTCCGTGCTGTGGCCGAGCCTGGCATCCAACTCAAGGCTGTG
AAGTCTTCCACAGGCCCCGGGGAACAGCTGCGGAACGCTCTGTGGCATACAGGAGACACA
GAGTCCCAGGTGCGGCTGCTGTGGAAGGACCCGCGAAACGTGGGTTGGAAGGACAAGAAG
TCCTATCGTTGGTTCCTGCAGCACCGGCCCCAAGTGGGCTACATCAGGGTGCGATTCTAT
GAGGGCCCTGAGCTGGTGGCCGACAGCAACGTGGTCTTGGACACAACCATGCGGGGTGGC
CGCCTGGGGGTCTTCTGCTTCTCCCAGGAGAACATCATCTGGGCCAACCTGCGTTACCGC
TGCAATGACACCATCCCAGAGGACTATGAGACCCATCAGCTGCGGCAAGCCTAG
Protein Properties
Number of Residues 757
Molecular Weight 82859.9
Theoretical pI 4.16
Pfam Domain Function
Signals
  • 1-20
Transmembrane Regions
  • None
Protein Sequence 
>Cartilage oligomeric matrix protein
MVPDTACVLLLTLAALGASGQGQSPLGSDLGPQMLRELQETNAALQDVRELLRQQVREIT
FLKNTVMECDACGMQQSVRTGLPSVRPLLHCAPGFCFPGVACIQTESGARCGPCPAGFTG
NGSHCTDVNECNAHPCFPRVRCINTSPGFRCEACPPGYSGPTHQGVGLAFAKANKQVCTD
INECETGQHNCVPNSVCINTRGSFQCGPCQPGFVGDQASGCQRRAQRFCPDGSPSECHEH
ADCVLERDGSRSCVCAVGWAGNGILCGRDTDLDGFPDEKLRCPERQCRKDNCVTVPNSGQ
EDVDRDGIGDACDPDADGDGVPNEKDNCPLVRNPDQRNTDEDKWGDACDNCRSQKNDDQK
DTDQDGRGDACDDDIDGDRIRNQADNCPRVPNSDQKDSDGDGIGDACDNCPQKSNPDQAD
VDHDFVGDACDSDQDQDGDGHQDSRDNCPTVPNSAQEDSDHDGQGDACDDDDDNDGVPDS
RDNCRLVPNPGQEDADRDGVGDVCQDDFDADKVVDKIDVCPENAEVTLTDFRAFQTVVLD
PEGDAQIDPNWVVLNQGREIVQTMNSDPGLAVGYTAFNGVDFEGTFHVNTVTDDDYAGFI
FGYQDSSSFYVVMWKQMEQTYWQANPFRAVAEPGIQLKAVKSSTGPGEQLRNALWHTGDT
ESQVRLLWKDPRNVGWKDKKSYRWFLQHRPQVGYIRVRFYEGPELVADSNVVLDTTMRGG
RLGVFCFSQENIIWANLRYRCNDTIPEDYETHQLRQA
GenBank ID Protein 602450
UniProtKB/Swiss-Prot ID P49747
UniProtKB/Swiss-Prot Entry Name COMP_HUMAN
PDB IDs Not Available
GenBank Gene ID L32137
GeneCard ID COMP
GenAtlas ID COMP
HGNC ID HGNC:2227
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A, Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA, Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M, Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V, Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S, Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D, Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS, DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA, Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM, Lucas SM: The DNA sequence and biology of human chromosome 19. Nature. 2004 Apr 1;428(6982):529-35. [PubMed:15057824 ]
  3. Chen FH, Herndon ME, Patel N, Hecht JT, Tuan RS, Lawler J: Interaction of cartilage oligomeric matrix protein/thrombospondin 5 with aggrecan. J Biol Chem. 2007 Aug 24;282(34):24591-8. Epub 2007 Jun 22. [PubMed:17588949 ]
  4. Di Cesare PE, Chen FS, Moergelin M, Carlson CS, Leslie MP, Perris R, Fang C: Matrix-matrix interaction of cartilage oligomeric matrix protein and fibronectin. Matrix Biol. 2002 Aug;21(5):461-70. [PubMed:12225811 ]
  5. Newton G, Weremowicz S, Morton CC, Copeland NG, Gilbert DJ, Jenkins NA, Lawler J: Characterization of human and mouse cartilage oligomeric matrix protein. Genomics. 1994 Dec;24(3):435-9. [PubMed:7713493 ]
  6. Briggs MD, Hoffman SM, King LM, Olsen AS, Mohrenweiser H, Leroy JG, Mortier GR, Rimoin DL, Lachman RS, Gaines ES, et al.: Pseudoachondroplasia and multiple epiphyseal dysplasia due to mutations in the cartilage oligomeric matrix protein gene. Nat Genet. 1995 Jul;10(3):330-6. [PubMed:7670472 ]
  7. Chen H, Deere M, Hecht JT, Lawler J: Cartilage oligomeric matrix protein is a calcium-binding protein, and a mutation in its type 3 repeats causes conformational changes. J Biol Chem. 2000 Aug 25;275(34):26538-44. [PubMed:10852928 ]
  8. Thur J, Rosenberg K, Nitsche DP, Pihlajamaa T, Ala-Kokko L, Heinegard D, Paulsson M, Maurer P: Mutations in cartilage oligomeric matrix protein causing pseudoachondroplasia and multiple epiphyseal dysplasia affect binding of calcium and collagen I, II, and IX. J Biol Chem. 2001 Mar 2;276(9):6083-92. Epub 2000 Nov 17. [PubMed:11084047 ]
  9. Mann HH, Ozbek S, Engel J, Paulsson M, Wagener R: Interactions between the cartilage oligomeric matrix protein and matrilins. Implications for matrix assembly and the pathogenesis of chondrodysplasias. J Biol Chem. 2004 Jun 11;279(24):25294-8. Epub 2004 Apr 9. [PubMed:15075323 ]
  10. Chen FH, Thomas AO, Hecht JT, Goldring MB, Lawler J: Cartilage oligomeric matrix protein/thrombospondin 5 supports chondrocyte attachment through interaction with integrins. J Biol Chem. 2005 Sep 23;280(38):32655-61. Epub 2005 Jul 28. [PubMed:16051604 ]
  11. Koelling S, Clauditz TS, Kaste M, Miosge N: Cartilage oligomeric matrix protein is involved in human limb development and in the pathogenesis of osteoarthritis. Arthritis Res Ther. 2006;8(3):R56. Epub 2006 Mar 15. [PubMed:16542502 ]
  12. Liu CJ, Kong W, Xu K, Luan Y, Ilalov K, Sehgal B, Yu S, Howell RD, Di Cesare PE: ADAMTS-12 associates with and degrades cartilage oligomeric matrix protein. J Biol Chem. 2006 Jun 9;281(23):15800-8. Epub 2006 Apr 12. [PubMed:16611630 ]
  13. Gagarina V, Carlberg AL, Pereira-Mouries L, Hall DJ: Cartilage oligomeric matrix protein protects cells against death by elevating members of the IAP family of survival proteins. J Biol Chem. 2008 Jan 4;283(1):648-59. Epub 2007 Nov 8. [PubMed:17993464 ]
  14. Hecht JT, Nelson LD, Crowder E, Wang Y, Elder FF, Harrison WR, Francomano CA, Prange CK, Lennon GG, Deere M, et al.: Mutations in exon 17B of cartilage oligomeric matrix protein (COMP) cause pseudoachondroplasia. Nat Genet. 1995 Jul;10(3):325-9. [PubMed:7670471 ]
  15. Ballo R, Briggs MD, Cohn DH, Knowlton RG, Beighton PH, Ramesar RS: Multiple epiphyseal dysplasia, ribbing type: a novel point mutation in the COMP gene in a South African family. Am J Med Genet. 1997 Feb 11;68(4):396-400. [PubMed:9021009 ]
  16. Susic S, McGrory J, Ahier J, Cole WG: Multiple epiphyseal dysplasia and pseudoachondroplasia due to novel mutations in the calmodulin-like repeats of cartilage oligomeric matrix protein. Clin Genet. 1997 Apr;51(4):219-24. [PubMed:9184241 ]
  17. Briggs MD, Mortier GR, Cole WG, King LM, Golik SS, Bonaventure J, Nuytinck L, De Paepe A, Leroy JG, Biesecker L, Lipson M, Wilcox WR, Lachman RS, Rimoin DL, Knowlton RG, Cohn DH: Diverse mutations in the gene for cartilage oligomeric matrix protein in the pseudoachondroplasia-multiple epiphyseal dysplasia disease spectrum. Am J Hum Genet. 1998 Feb;62(2):311-9. [PubMed:9463320 ]
  18. Ikegawa S, Ohashi H, Nishimura G, Kim KC, Sannohe A, Kimizuka M, Fukushima Y, Nagai T, Nakamura Y: Novel and recurrent COMP (cartilage oligomeric matrix protein) mutations in pseudoachondroplasia and multiple epiphyseal dysplasia. Hum Genet. 1998 Dec;103(6):633-8. [PubMed:9921895 ]
  19. Loughlin J, Irven C, Mustafa Z, Briggs MD, Carr A, Lynch SA, Knowlton RG, Cohn DH, Sykes B: Identification of five novel mutations in cartilage oligomeric matrix protein gene in pseudoachondroplasia and multiple epiphyseal dysplasia. Hum Mutat. 1998;Suppl 1:S10-7. [PubMed:9452026 ]
  20. Susic S, Ahier J, Cole WG: Pseudoachondroplasia due to the substitution of the highly conserved Asp482 by Gly in the seventh calmodulin-like repeat of cartilage oligomeric matrix protein. Hum Mutat. 1998;Suppl 1:S125-7. [PubMed:9452063 ]
  21. Unger S, Korkko J, Krakow D, Lachman RS, Rimoin DL, Cohn DH: Double heterozygosity for pseudoachondroplasia and spondyloepiphyseal dysplasia congenita. Am J Med Genet. 2001 Nov 22;104(2):140-6. [PubMed:11746045 ]
  22. Mabuchi A, Haga N, Ikeda T, Manabe N, Ohashi H, Takatori Y, Nakamura K, Ikegawa S: Novel mutation in exon 18 of the cartilage oligomeric matrix protein gene causes a severe pseudoachondroplasia. Am J Med Genet. 2001 Nov 22;104(2):135-9. [PubMed:11746044 ]
  23. Czarny-Ratajczak M, Lohiniva J, Rogala P, Kozlowski K, Perala M, Carter L, Spector TD, Kolodziej L, Seppanen U, Glazar R, Krolewski J, Latos-Bielenska A, Ala-Kokko L: A mutation in COL9A1 causes multiple epiphyseal dysplasia: further evidence for locus heterogeneity. Am J Hum Genet. 2001 Nov;69(5):969-80. Epub 2001 Sep 14. [PubMed:11565064 ]